Multifocal hemangioendothelioma of the lumbar spine and response to surgical resection and radiation

Abstract

Background context: Epithelioid hemangioendothelioma rarely occurs in the lumbosacral spine, with very few case reports of spinal hemangioendothelioma in the literature. There is variability in aggressiveness of these lesions without established treatment guidelines.

Purpose: The aim was to present a case of epithelioid hemangioendothelioma in the lumbar spine, including magnetic resonance imaging (MRI) findings, which rapidly progressed over a 2-month period as regional multifocal lumbosacral spinal lesions with epidural extension causing severe spinal canal stenosis.

Study design/setting: This was a case report in a university hospital setting.

Patient sample: The sample included an otherwise healthy adult male with low back pain.

Methods: Multimodality imaging was performed to help with diagnosis and management including computed tomography, MRI, and positron emission tomography (PET). The patient was treated by embolization, L5 corpectomy and L4-S1 stabilization, and radiation therapy. The diagnosis was confirmed by tissue biopsy.

Results: The patient initially presented with severe back and leg pain after a vertebroplasty for an L5 compression fracture at an outside hospital where biopsy was negative for malignancy. Magnetic resonance imaging showed diffuse abnormality of L5 with several smaller lesions in the sacrum. Due to progressive pain 2 weeks after the vertebroplasty, the patient underwent an L5 laminectomy, L4-S1 instrumented posterior fusion, and attempted partial corpectomy for stenosis. At this surgery, the L5 corpectomy was aborted owing to profound bleeding. Pathology was again negative for malignancy. Presumed to be an atypical hemangioma, the lesion was embolized before repeat surgery where the thecal sac was decompressed by partial L5 corpectomy. Biopsy at this time revealed a vascular neoplasm, with hemangioendothelioma not excluded. Approximately 2 months after the stabilization procedure, the patient had increasing pain and bilateral lower extremity weakness. Magnetic resonance imaging was performed and demonstrated marked local progression of disease with new multifocal lesions involving L4 through S2 vertebrae and new severe spinal canal stenosis. These lesions were subsequently treated with localized radiation therapy. Magnetic resonance imaging 2 months after radiation therapy showed significant regression of the epidural tumor although a new metastatic lesion was discovered at T6 vertebra.

Conclusions: Spinal hemangioendothelioma is a rare disease and can present in variable forms, including as a multifocal regional process--which may be mistaken for infection. Additionally, there are no standard treatment protocols for this entity. We present the extensive imaging and treatment of a single case of rapidly progressive lumbar epithelioid hemangioendothelioma, which to our knowledge has not been described with this multifocal appearance in the lumbar spine.

Keywords: Bone neoplasm; Epithelioid; Hemangioendothelioma; Imaging; Radiation; Spine; Surgery; Vascular; Vascular tumor.